Mendeloff E N, Huddleston C B
Department of Surgery, Washington University School of Medicine, St. Louis, MO, USA.
Semin Thorac Cardiovasc Surg. 1998 Apr;10(2):144-51. doi: 10.1016/s1043-0679(98)70009-7.
Pulmonary vascular disease in conjunction with either a previously repaired or an unrepaired congenital heart defect is the third most common indication for lung transplantation in the pediatric age range. Because scarcity of donor organs remains a critical issue and heart-lung donor blocks are becoming diminishingly available, efforts must be directed towards other options such as combining lung transplantation with correction of the underlying congenital heart defect. Certain defects like congenital pulmonary vein stenosis are eradicated by removal of the diseased lungs, whereas others such as complete atrioventricular canal and pulmonary atresia with ventricular septal defect require cardioplegic arrest of the heart and intracardiac repair in conjunction with the lung transplantation. A breakdown of this patient population into subgroups may be helpful both in thinking about the pathophysiology and in determining appropriate indications and timing of transplantation. Earlier studies from our center showed the high-risk nature and formidable undertaking of caring for this complex group of patients. Through continued experience, there has been gradual improvement in early outcomes. As with all other groups of lung transplantation patients, obliterative bronchiolitis remains the major deterrent to long-term survival.
与先前已修复或未修复的先天性心脏缺陷相关的肺血管疾病是儿童年龄范围内肺移植的第三大常见适应症。由于供体器官短缺仍然是一个关键问题，且心肺供体阻滞越来越难以获得，因此必须努力寻求其他选择，例如将肺移植与潜在先天性心脏缺陷的矫正相结合。某些缺陷，如先天性肺静脉狭窄，可通过切除患病肺脏来根除，而其他缺陷，如完全性房室通道和伴有室间隔缺损的肺动脉闭锁，则需要在心脏停搏的情况下进行心脏内修复并结合肺移植。将这一患者群体细分为亚组，可能有助于思考病理生理学以及确定合适的移植适应症和时机。我们中心早期的研究表明，照顾这一复杂患者群体具有高风险性质且任务艰巨。通过持续的经验积累，早期结果已逐渐改善。与所有其他肺移植患者群体一样，闭塞性细支气管炎仍然是长期生存的主要障碍。
